AW Morrow Gastroenterology and Liver Centre, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.
Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia.
ANZ J Surg. 2022 Oct;92(10):2425-2432. doi: 10.1111/ans.17948. Epub 2022 Aug 3.
Cystic fibrosis (CF) is a complex multiorgan disease, which often affects the gastrointestinal tract. With improved CF specific therapies and multidisciplinary management, patients with CF are now living longer with a median life expectancy of around 50 years. This increased life expectancy has resulted in corresponding increase in presentations of the CF patient with comorbid surgical conditions that were never important considerations. Investigations and management of these conditions, such as distal intestinal obstruction syndrome and colorectal cancer warrant good clinical understanding of the unique challenges that CF patients present including chronic immunosuppression, impaired respiratory function and their multi-organ dysfunction. The purpose of this review is to provide general surgeons with a contemporary update on the CF related surgical issues as they are likely to become increasingly involved in the care of these complex patients and form an integral part of the multidisciplinary team.
囊性纤维化(CF)是一种复杂的多器官疾病,常影响胃肠道。随着 CF 特异性治疗和多学科管理的改进,CF 患者的寿命现在更长,中位预期寿命约为 50 年。这种预期寿命的延长导致伴有合并症的 CF 患者就诊数量相应增加,这些合并症以前从未是重要的考虑因素。对于这些疾病的检查和管理,例如远端肠梗阻综合征和结直肠癌,需要很好地了解 CF 患者的独特挑战,包括慢性免疫抑制、呼吸功能受损和多器官功能障碍。本综述的目的是为普通外科医生提供有关 CF 相关手术问题的最新信息,因为他们可能会越来越多地参与这些复杂患者的治疗,并成为多学科团队的重要组成部分。